Frequency of reoperations in patients with Marfan syndrome

Sarah Geisbuesch1, Deborah Schray, Moritz S Bischoff

  • 1Department of Cardiothoracic Surgery, Mount Sinai Medical Center, New York, New York 10029, USA. sarah.geisbuesch@mountsinai.org

Abstract

Insights

Reoperation is common in Marfan syndrome patients undergoing aortic surgery, but mortality is low. Elective initial surgery leads to better outcomes and fewer reoperations compared to emergency procedures.

Area of Science:

  • Cardiovascular Surgery
  • Genetics
  • Aortic Diseases

Background:

  • Marfan syndrome is a genetic disorder affecting connective tissue, often leading to aortic complications.
  • Surgical intervention is frequently required for aortic root and ascending aorta issues in Marfan patients.
  • Reoperations are a significant consideration in the long-term management of these patients.

Purpose of the Study:

  • To analyze the patterns and outcomes of reoperations in patients with Marfan syndrome.
  • To compare the results of elective versus emergency initial aortic surgeries.
  • To identify factors influencing reoperation rates and mortality in this population.

Main Methods:

  • Retrospective study of 83 Marfan patients who underwent 155 aortic operations between 1985 and 2008.
  • Comparison of outcomes between patients with initial elective surgery (Group I) and emergency surgery (Group II).
  • Analysis included reoperation rates, types of aortic procedures, aortic segments replaced, and survival.

Main Results:

  • Overall, 81/83 patients required root/ascending aortic repair, with 36% undergoing reoperation.
  • Operative mortality was significantly lower for initial elective surgery (1.6%) compared to emergency surgery (9.0%).
  • Elective surgery patients had fewer reoperations, fewer aortic segments replaced, and a trend towards improved survival.

Conclusions:

  • Reoperation is common in Marfan syndrome patients after aortic surgery, but reoperative mortality is low.
  • Initial elective aortic root replacement in Marfan patients is associated with better long-term outcomes.
  • Prompt surgical intervention for significant aortic root dilatation in Marfan syndrome is recommended.